Department of Dermatology, Southampton General Hospital, University Hospitals Southampton NHS Foundation Trust.
Dermatoimmunology, Sir Henry Wellcome Laboratories, Clinical, Experimental Sciences, Faculty of Medicine, University of Southampton, Southampton, UK.
Curr Opin Allergy Clin Immunol. 2019 Aug;19(4):283-293. doi: 10.1097/ACI.0000000000000546.
Severe cutaneous adverse reactions (SCAR) are relatively uncommon but can be life-threatening. This review focuses on the nonanaphylactic (non-IgE-mediated) phenotypes of drug hypersensitivity, with specific reference to diagnosis and management of acute generalized exanthematous pustulosis (AGEP), drug reaction with eosinophilia and systemic symptoms (DRESS), Stevens-Johnson syndrome (SJS), and toxic epidermal necrolysis (TEN).
Here, we review recent guidelines on optimal supportive care as well as publications of interventional treatment for SJS/TEN, including various immunomodulating therapies, and management strategies for severe ocular disease with the use of amniotic membrane transplantation. In DRESS, long-term autoimmune sequelae are increasingly recognized and modify strategies for treatment of the acute episode. If the causative drug is not apparent from careful inspection of the drug exposure history, in-vitro diagnostics, HLA testing and skin testing before drug challenge testing may be considered and we present an algorithm for investigation of complex cases.
Careful phenotypic analysis of the increasingly complex recognized patterns of SCAR facilitates the enhancement in our understanding of T-cell mediated drug hypersensitivity and allows the improvement of in-vitro diagnostic testing to minimize patient exposure to test substances in all but a very limited number of cases, thereby enhancing safety.
严重皮肤不良反应（SCAR）相对少见，但可能危及生命。本综述重点介绍药物超敏反应的非过敏（非 IgE 介导）表型，特别参考急性全身性发疹性脓疱病（AGEP）、药物伴嗜酸性粒细胞增多和全身症状（DRESS）、史蒂文斯-约翰逊综合征（SJS）和中毒性表皮坏死松解症（TEN）的诊断和管理。
在这里，我们回顾了最近关于最佳支持性护理的指南以及 SJS/TEN 的干预治疗的出版物，包括各种免疫调节治疗，以及使用羊膜移植治疗严重眼部疾病的管理策略。在 DRESS 中，越来越多的人认识到长期自身免疫后遗症，并改变了急性发作的治疗策略。如果从仔细检查药物暴露史、体外诊断、HLA 测试和药物挑战测试前的皮肤测试中不能明显看出致病药物，可考虑进行体外诊断测试，并提出了用于复杂病例的调查算法。
对越来越复杂的 SCAR 表型进行仔细的表型分析，有助于我们加深对 T 细胞介导的药物超敏反应的理解，并改进体外诊断测试，以尽量减少患者接触测试物质的机会，除了极少数情况下，从而提高安全性。
